Immunophenotypic and molecular features, clinical outcomes, treatments, and prognostic factors associated with subcutaneous panniculitis‐like T‐cell lymphoma

Abstract

BACKGROUND Subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL) is an uncommon type of skin lymphoma. The natural history, optimal treatment strategy, and prognostic factors associated with this malignancy are not well defined. METHODS The authors performed a systematic analysis of all patients with SPTCL reported on in the English‐language medical literature, with emphasis on specific clinical features, experiences involving the use of radiotherapy and systemic agents, and prognostic factors predictive of treatment response and clinical outcome. RESULTS One hundred fifty‐six patients with SPTCL were identified in the literature. Hemophagocytic syndrome (HPS) was a presenting feature in 37% of patients, and > 90% of patients required treatment at diagnosis. Prednisone was used frequently as initial therapy in patients who had less aggressive disease at presentation; however, durable complete remissions (CR) were infrequent. Anthracycline‐based chemotherapy regimens were the most commonly used and most effective systemic treatment options, producing long‐term CR in ∼30% of patients. Among patients who received high‐dose chemotherapy and stem cell transplantation (HDT‐SCT) for refractory or recurrent disease, 92% achieved CR, with a median response duration of ≥ 14 months. The presence of HPS at diagnosis and expression of the γ/δ T‐cell receptor (TCR) by tumor cells were associated with poor survival, whereas age was not. After a median follow‐up of 24 months, 48% of patients died of disease. The median survival duration was 27 months. CONCLUSIONS SPTCL has an aggressive natural history. Nonetheless, a subgroup of patients with SPTCL can have long‐term disease remission following anthracycline‐based initial therapy or subsequent HDT‐SCT. HPS and the TCR phenotype may be useful prognostic markers for patients with this malignancy. Cancer 2004. © 2004 American Cancer Society.

Subcutaneous Panniculitis- like T-Cell Lymphoma: Report of two cases

“…High dose chemotherapy and stem cell transplantation is usually considered in primary refractory or recurrent cases. [4,6] The response is variable but usually the prognosis is unfavorable in the presence of constitutional symptoms, cytopenia, multiple site involvement and associated haemophagocytic syndrome (HPS) [6,9,10]. In conclusion, SPTCL represents a distinct clinicopathologic entity of clonal, cytotoxic T-cell lymphomas derived from αβ T cells.…”

Linfoma subcutâneo de células T paniculite-símile

“…Nesse útimo quadro, o LSCTPS frequentemente é associado à síndrome hemofagocítica. 5,6 A histopatologia é caracterizada por padrão paniculitesímile com envolvimento neoplásico do tecido subcutâneo. Essa infiltração do subcutâneo por células T pleomórficas e macrófagos benignos mimetiza paniculite lobular.…”

“…As células malignas são predominantemente células T citotóxi-cas com expressão αβ ou γδ dos receptores das célu-las T (TCR). 6 As manifestações clínicas inespecíficas do LSCTPS podem dificultar e postergar o seu diagnósti-co, para o qual o exame histopatológico e o imunoistoquímico são fundamentais. O eritema nodoso e as paniculites com distintas etiopatogenias são seus principais diagnósticos diferenciais.…”

Subcutaneous panniculitis‐like T‐cell lymphoma with extracutaneous dissemination demonstrated on FDG PET/CT

“…[1] It is a rare lymphoma (<1% non-Hodgkin lymphoma) that may affect younger patients (median age 35 years) and has an association with autoimmune disease. Dissemination to lymph nodes and other organs on initial presentation is very unusual.…”